Ranting and Raving

In fairness to everyone I am going to put a "caution label" on this post, both for length and content. In short, the following is a letter I wrote this morning to Max's Nurse Case Manager at his Medicaid Plan regarding Max's post-op care in Phoenix.

It took me many tries and a lot of editing to get this letter to a place that was not full of rage and accusations. It was by no means a letter that was easily written but it was a letter that NEEDED to be written. We are extremely fortunate to be able to take Max to a place like Hopkins for medical care, not all families are that blessed and it is important that the State of Arizona recognize that the pediatric health care system in this State is broken and children are paying the price.

I have eliminated names to protect both the innocent as well as the guilty and I ask all of you to accept this as my attempt to state what is so clearly obvious to many of us who parent chronically ill children.....plus it was also GREAT therapy for me!

Good Morning P –

I am writing this to you because of deep concerns we have regarding Max’s post-op care here in Phoenix.

As you know Max is just over three weeks post –op for his aortic valve replacement at Hopkins. We returned just this weekend due to a protracted and rocky recovery. Before discharging us Hopkins reviewed several conditions for discharge home with us, they are as follows:

1. We had to have a cardiology appointment no later than Monday or Tuesday of this week. These follow ups should continue as frequently as weekly if need be due to the number of post-op issues Max experienced. This should include a rather vigorous echo schedule due to marked (measured as moderately diminished function by echo twice at Hopkins) post-op heart failure.
2. Coumadin checks would have to be handled on a minimum of an every other day basis as Max was diagnosed with HIT (heparin-induced thrombocytopenia) and therefore could not get out of range due to the fact that heparin or lovenox would not be a “rescue” option for Max.

These conditions made logical sense to us as parents and we agreed to follow up with our cardiologist here in Phoenix. In the meantime, Hopkins Cardiology and Hematology contacted their counterparts here (I had given them Dr. P’s name as that was who Dr. N suggested during our visit with him pre-op), all paperwork was faxed at least once to the office here, including a very detailed coumadin protocol devised especially for Max by his hematologist at Hopkins.

Upon our return to Phoenix we needed to have an INR check on Sunday so Hopkins called the PCH ER to arrange this, this option was chosen because as it was a Sunday there would be few options for a blood draw at any other facility. Obviously an ER visit was not optimum (and certainly not cost-efficient) but it was the only option. It should be noted that Max’s INR was 3.0 on Sunday, just at the high end of the suggested 2.0 to 3.0 range. The PCH ER Attending called Hopkins with this info and arrangements were made regarding dosing by them. When I spoke with Hopkins on Sunday they confirmed with me that PCH Cardiology would be running another INR on Tuesday during our appointment, they once again reminded me of the importance of keeping on top of this.

Therefore, on Tuesday we went to PCH for our appointment with Dr. R as Dr. P would be out of the office this week. Before I review this appointment and our concerns with Max’s care during this appointment I really, really want to stress that I went to this appointment full of optimism that we would have a good source of care for Max in Phoenix. This optimism, unfortunately was quickly dashed as we waited for nearly 50 minutes for the doctor to come into the exam room. We understand just how overworked doctors can be so although this wait was not great, especially for a ten year old little boy, we assumed that once Dr. R came into the room we would receive an acceptable amount of his time reviewing Max and his treatment. This was decidedly not the case; Dr. R came in, listened to Max’s heart commented on the “tick” of the valve and suggested that an echo and coumadin check could be done in a month. When I questioned the safety of this and explained what Hopkins requested I was told that their protocol seemed “excessive” (direct quote), I pushed for a script for an INR and when asked I was told not to do it at the Hospital because a Sonora Quest draw would be quicker. I let Dr. R know that Hopkins was waiting for an INR number and asked him to call both myself and Hopkins later in the day with Max’s number. Dr. R left the room and said he would be back shortly with the script, he did not return but rather sent his assistant in with the script. In total our appointment with the doctor lasted under a minute and consisted of nothing other than an EKG and a quick listen to Max’s heart. In short, I feel that this was not a thorough post-op appointment on any level and the suggestion that coumadin checks could be done on a monthly basis for a highly complex child who had barely been in consistent therapeutic range for a short number of days was medically negligent at best.

When my husband took Max to Sonora Quest shortly after the appointment he was told that Dr. R would not have any results for at least another 36 hours. Obviously this would be outside of the protocol that we were given asking for a number and correlated dosage change if necessary yesterday. As a concerned mother I call Max’s hematologist at Hopkins to ask him how comfortable they felt with this wait, I was told that it would not be acceptable to wait for this mainly for the reasons I have outlined above. Arrangements were quickly made via telephone between Hopkins and Mercy Gilbert Hospital’s ER to do a stat draw. Again not optimal but at that point everyone’s hands were tied. We are incredibly thankful that we did this because Max was under therapeutic range at 1.9 and needed a supplemental dose of coumadin. Please note that all of this was taking place at midnight East Coast time but that did not deter the Hopkins doctor from researching on-line phone numbers he could use to try to reach Sonora Quest to see if they could give him a verbal result, Hopkins is also continuing to research a feasible long term option for Max here in Phoenix. Baltimore is doing this from 2500m miles away.

This morning I called Dr. N’s office and spoke to his assistant E. I explained what had transpired and asked if Dr. N could work with Hopkins on the coumadin levels. E asked me who we had seen yesterday and told me that she would contact Dr. N to see if he wanted to see us or would just give me a call. Either way she would get back to me in an hour or so. A short time later Dr. R called me back, obviously prompted by my conversation with E, he clearly stated that he was unsure of why I did what I did last evening and why Hopkins was using such a stringent protocol for Max because he did not agree with it. I politely told him that this was my son and I much preferred “stringent” and safe as opposed to any other option. This is indeed the life of a child. He felt that two draws yesterday was not “great”, I agreed with him that it wasn’t but sometimes “not great” is necessary. He emphatically reiterated to me that with the type of valve (On-X) Max was given in an aortic position he would not follow this protocol but that he would look into a home monitor as I requested yesterday and at finding out if Mercy Gilbert’s Coumadin Clinic would be a good option for Max going forward, the later of these two was mostly because I told him Hopkins was doing so and I feel he didn’t want to look as if he wasn’t trying.

Coincidentally as I am typing this to you Dr. R called me back to tell me that Mercy Gilbert’s outpatient lab has a two hour turnaround time and would therefore be the better option. He also told me that he had spoken to Dr. H at Hopkins who used the same protocol they did here in Phoenix, twice weekly checks. I find this strange because just yesterday Dr. R told us monthly checks were fine – how did he go from monthly to twice weekly draws as his protocol? I told him that, yes, we were told that we could go down in the number/frequency of INR checks once everyone was very comfortable that Max was consistently in range, as a result I suggested that we (he and I) were going to have to accept that we disagreed in how Max’s management was best served. He asked me who I wanted to have follow Max’s level and I told him I felt at this time Hopkins would be best suited to follow-up, especially because they are happy to do so. I did not exclude that this could change once everyone is convinced we are safely in range. I am not in any way looking to exclude the doctors here in Phoenix from Max’s care, obviously we need local doctors on “Max’s Team”, he asked who I would follow up with here as Max’s cardiologist. I told him that I would be using Dr. P and he said he would pass on the information he had to him.

Please forgive the lengthiness of my e-mail and know that I am no way trying to “cause problems”, I truly looked forward to forging a good working relationship with the doctors here in Phoenix. I desperately needed “peace of mind” that my son would have great local care. At the same time I feel that it is incidents such as these that have driven us to seek care for Max out of state at Johns Hopkins. I know that many people have been critical of us for this but we are merely acting as concerned and responsible parents, I can’t imagine any parent not doing this for their child.

Thanking you in advance for taking the time to read this.

Patricia

Welcome Home Max!

We're home and although we are all still groggy and adjusting to the time difference and the thermometer on our patio is reading 115 degrees there truly is NO PLACE like home! Max is completely back in his element enjoying another phase of "post surgery attention". During a quick Costco run yesterday Max's "Costco Gang" presented him with a cake to celebrate his triumphant return, he was so happy to see everyone and show them his scar. Not sure how many folks were really that interested in seeing it but they were flashed all the same......

We are settling in and trying to get used to our new "normal". This means trying to convince Max that his incision is still too fresh to play baseball, swim, or otherwise run around like a maniac. Of course high levels of blood thinners are also not conducive to jumping off the furniture but we're having a hard time convincing Max of this. While part of me wants to wrap him up in bubble wrap and duct tape him to the sofa I have to admit I love seeing a bit of the old Max start to peak through.

We are also busy trying to fine tune the new medicine schedule, if we thought Max was on a ton of meds before the list has only grown exponentially (I wish I had thought to bring home one of the nurses with me, I hate dosing meds!). This week we'll also have a doctor's appointment or two and certainly the dreaded coumadin level checks so check back for updates!

Hopkins Wrap Up

As I sit here looking around our room, suitcases piled high, Max sleeping next to me, it finally feels real....the roller coaster has slowed to a stop and the terrifying ride is over....at least for now. And at the moment "now" is all that matters.

Max's blood work this morning looked great. Because he's had a few consecutive days of being in "range" with his coumadin they are giving us tomorrow off, which means we don't need to go to Hopkins' ER (only the ER draws blood on Saturdays) in the morning for another draw. However it is still too early in Max's therapy to go more than one day without checking his levels so bright and early Sunday morning you can find us at Phoenix Children's ER having a check. Max's doctor here has already called PCH with the order so as long as nothing gets screwed up on the AZ end we shouldn't have to spend too much of our first day home in a hospital. But who really cares? At least we'll be home!

Before I go back to packing (or at least watching Michele pack) I want to thank everyone who sent messages, cards and presents, and even visited Max. It meant so much to him, and us, to know that everyone at home was thinking of him and praying for him to get well.

I guess there's just one thing left to say......Arizona here we come!

Hopkins - Post-Op Day Sixteen.....And We're Outta Here!

Sweet Sixteen. It took nearly three weeks but we've finally managed to get ourselves discharged!

We had been told yesterday that we were on the "likely to be discharged" list (one of the residents even showed me the list) but our superstitions NEVER allow us to utter the "H" word (home) before we're actually outside the four walls of the hospital. As any parent of a chronically ill kiddo will tell you the mere mention of the "H" word automatically buys you more hospital time.

As of this morning the doctors felt that Max was stable enough both with his coumadin levels and fever/infection status that there was no reason not to discharge us with returns to the outpatient clinic both tomorrow and Saturday morning. We will fly home Saturday evening and will perhaps need to have blood levels drawn at our local ER on Sunday and Monday before we see our AZ cardiologist on Tuesday. But who cares? We're going "H" (still can 't say the word until I'm unlocking my front door)!!!

We want to thank everyone who has prayed so hard with us these last few weeks for Max's successful surgery and full recovery. We firmly believe that these prayers guided all of Max's care, from the surgeon's hands to the ICU doctors and nurses and everyone else who cared for Max. Miracles do not come lightly and they certainly do not come without prayer. Thank you everyone from the bottom of our hearts, today we brought our baby "H"!!!

Today's pics:

Max on "Clown" TV

Spiffy Hat

Finally! Making a break for it!

Outside the double doors, first dose of fresh air in nearly three weeks!

Leaving open-heart surgery #4 behind.

Max arriving at our Baltimore timeshare. Doesn't everyone walk the streets in their brand new Monster Truck PJs?

Welcome home Max!

Hopkins - Post-Op Day Fifteen

Another day of sitting around and waiting. Doctors come in, doctors go out, in, out, in, out and another day passes. We're pretty much in a holding pattern waiting to see what Max's fevers will do, figuring out if there's an infection worth treating or not, and of course, getting his coumadin at a stable "therapeutic range". It always seems like it's the "little things" at the end that take up the most time, probably because at this point everything seems too long.

And while the time crawls and home seems painfully far away we know that there are many excellent doctors and speciality services all working diligently to make sure that Max and his shiny new valve are safe and healthy. I simply CANNOT imagine being anywhere other than Hopkins. Everyone takes such care with Max and they listen to us and our questions and it is a true dialogue, together as a team we are making decisions and mapping Max's plan of care for the next few months. Physicians that listen to and learn from parents....believe me, you DON'T see that very often at all!

Today's Pics:

Hospital BINGO!!!

Did he really just win University of Maryland boxers?

Hopkins - Post-Op Day Fourteen

Two weeks ago today almost to the hour Max was whisked past us going from the OR to the PICU. Since then so much has happened, the days are blurs, our minds are foggy. But today as I watched Max whip around the 4th floor going in and out of the playroom, even going down to the cafeteria with Ellie I almost forgot the nightmarish post-op week and a half in the PICU. The ventilator, the diminished heart function, the fevers, it all seems so long ago even if in reality it was just last week. As Max's physical pain has eased so too has our intense emotional pain, we never really forget any of it but God has blessed us with the ability to reflect on the good that has come from all of this and not dwell on what went wrong.

Today's pics:

A gift from Gabby

A rare occasion caught on film!

Hopkins - Post-Op Day Thirteen

Today was pretty much just a sit and wait day. Sit and wait for the doctors. Sit and wait for lab work, x-rays, and echo cardiograms. Sit and wait for answers.

Today's answers were varied. The best answer we received, hands down, was the results of Max's echo which showed that his heart function had returned to "good". Just days ago we were told that it was moderately diminished and it would take pretty aggressive therapy and time to return if it returned at all. There were no guarantees. And today we received another "Max Miracle", proof that doctors are human and diagnoses are often just their best guesses.

The results of Max's cultures are starting to trickle in and it seems like he may have a fungal UTI. Not a great answer but at least it may explain the fevers. The only problem now is that any of the drugs in the anti-fungal family would interact with his coumadin levels so they've taken a second culture to be sure what and if we need to treat. Just some more waiting, unfortunately we're getting good at it.

Today's pic, Max doing his Physical Therapy.....the Wii!!

Hopkins - Post-Op Day Twelve

Today we are officially in the hospital longer than we were for Max's surgery in 2007 and we thought that stay went on forever......

The hot topic (no pun intended) of the day wasn't really the fevers anymore, that fire drill seems to have past. Today's issue centers around Max's lab results which indicate that his coumadin (blood thinner) levels are extremely high -- obviously this puts him at an increased bleeding risk so caution has been the order of the day.

Yesterday we had a great visit from some AZ friends who are vacationing in the area, it was so nice to see some familiar faces. Bob and Terri we can't tell you how much your visit meant to all of us!

A visit from home - Max and his football buddy Chris

Tonight's prayers: please pray that Max will have no bleeding issues whatsoever, that his blood work will show no sign of infection, and that the fevers will subside.

Stay tuned.....

Hopkins - Post-Op Day Eleven

I have officially jinxed us. Max has a fever (which he has had on and off since surgery) and the floor doctors don't feel comfortable about not aggressively looking for a source. Because of his high heart rate, low blood pressure, and impressively high white blood cell count they are culturing both blood and urine and while I appreciate their thoroughness and understand its importance, especially in light of the fact that Max just had an artificial part put in his heart, it is difficult to not be frustrated. All we want to do is go home and this won't help our case.

It's been a rough day all around for us but not as much as it has been for Max. Once he left the PICU his central line (sort of a "super IV") had to be removed which means all his many labs have to be drawn each time. That's a lot of needle sticks and if you throw in the twice a day shots he now needs it has become just too much for this little boy. He is tired and cranky and wants nothing more to do with this whole situation. Who can really blame him? As hard as this whole thing has been for us it is nothing compared to what Max has had to endure and it is heart breaking to watch him sit silently in his bed and cry quiet tears as someone sticks, pokes or prods him.

So tonight please pray for Max. Pray that all of his tests will come back negative for any type of infection, that the fevers will subside, and that his heart rate and blood pressure numbers will even out.

And now a few pics from Max's day:

Walking the Halls

Ready for Bed

Where are we?

I've had a few requests from folks asking for the address of our Baltimore "timeshare". So here goes:


Children's House at Johns Hopkins
ATTN: Max Marangella, Room 204
1915 McElderry St
Baltimore, Md 21205


Have a great Saturday!

Hopkins - Post-Op Day Ten

Ladies and gentlemen I am happy to report that the Marangella's have been kicked out of the PICU....and it wasn't my fault! Last night the attending doc came in to tell me that Max was officially "the least sick" child in the ICU and they needed his bed. The least sick? Less than 48 hours ago he was definitely in the top five!

This morning they packed us up and shoved us towards the elevators to send us down to the fourth floor. Private rooms, SCORE! Michele insists he heard the nurses cheering as the door swung close behind me. I decided not to take it personally until we got to the "floor" and our night nurse immediately remembered me as the mom who gave a resident hell during her very first week of residency for being insensitive and refusing to take what we as parents were telling her into consideration. Evidently this episode is a classic and recounted over and over again amongst the nurses. I'd like to think I taught this resident something because four years later she's Chief Resident at Hopkins, the number one hospital in America.

As for Mr. Max, he is doing so well that I don't want to jinx ourselves too much but I cannot believe how well he's hopping in and out of bed and chasing down the hallways now that he is no longer tethered to an IV pole. The physical therapist admitted that we might not need her after all! Now all we have to do is get Max's blood thinner values into "therapeutic range" and we might just be able to see the light at the end of the tunnel.

Stay tuned....

Hopkins - Post-Op Day Nine

Today was a day of firsts: first hallway walk, first sitting up in a chair, and first SUBWAY Spicy Italian sub! It is so hard to believe that just 48 hours ago Max was so sick that the doctors really didn't know how to safely extubate him. Looking at him today I almost forgot that earlier in the week his heart and lungs were both not functioning properly. Max never ceases to amaze me, he can go from super sick to his typical goofy self in a matter of hours. It is nothing less than a miracle and we are so truly blessed and thankful!

Because Max has spent nine solid days in bed they had him start working with a physical therapist today as well as with the Pain Management Team so that he can be safely weaned from all the narcotics they have been snowing him with. I now know how Lindsey Lohan's mother must feel, my ten year old is a candidate for drug rehab!

Praying that each day Max gets stronger and closer to the door. We all want nothing more than to be home!

Stay tuned.....

Hopkins - Post-Op Day Eight

Max is holding his own! He continues to breathe well with the tube out and bi-pap on! It truly feels like a miracle, we must admit that we were fearing for the worst only because we have seen so much pain and suffering around us in the close quarters that is the PICU at Hopkins. Sleep deprivation is a horrible, horrible thing and it allows the mind to wander to places it should not go.

Last night as the night nurses came on many of them ran up and hugged and kissed Max as they were genuinely thrilled to see his breathing tube out (for those of you who have asked, yes there is still a nurse or two who will speak to me). During evening rounds tonight the doctor that I love so much was super excited to play the hero and allow Max to start drinking ginger ale, it is the first thing that has passed his lips since last week Monday. The look of sheer delight on his face behind his mask was priceless! There is hope that if he continues to do well he might be able to eat tomorrow. This is a very, very good thing because Max begged me for a bowl of cereal tonight -- he must be desperate because he HATES cereal!

None of today's successes guarantees that we are completely out of the woods. We still have to see if Max's heart will regain some of the function it lost due to surgery and the doctors have to find Max's "sweet spot" with blood thinners -- enough to not form clots but not have bleeding issues. Please pray that these things happen safely for Max.

Stay tuned.....

Hopkins - Post-Op Day Seven

Today marks one week since Max's surgery. Many things have gone right, many have gone wrong. After seven days we should no longer be in the PICU but rather "out on the floor" in a regular room. But Max has always followed his own plan and this is no exception. Today his plan included getting his breathing tube out!!

This morning the Attending doctor came to Max's bed very early, before rounds, to tell me that the AM x-ray looked very good. To quote her, "This is as good as it gets." Their plan was to extubate right after rounds early this morning. Their plan, not Max's. Shortly after the doctor's visit Max had had enough and attempted once again to push the tube out on his own with his tongue. This sent him off into a mini panic attack but not quite as bad as yesterday's. Because there were several other PICU emergencies (in the last 24 hours there have been several very sick children, please pray for them and their families) none of the doctors were able to get to Max's bed to get the tube out and thus the nurses had to sedate him again. Obviously a sedated child cannot be extubated so we were back to waiting. This last week there has been too much waiting especially for some very impatient parents but in the end I guess good things really do come to those who wait.

Once Max started to wake up this afternoon I walked the halls and rounded up the doctors myself, I have not felt the nurses' love for this lack of respect for the PICU rules and pecking order but this isn't a Miss Congeniality contest and I wasn't looking to make friends but rather do what's best for my son. Parenting a chronically ill child is tough work and sometimes being tough is the only way to get the job done. And done it is! The tube is out! Max and many, many prayers have done it! The tube is gone and we are now on bi-pap again until our next x-ray at about 8 or 9 o'clock, that x-ray will show us were Max is breathing on his own. Please, please pray for success, psychologically Max needs this success in order to make his plan work.

Stay tuned.....

Hopkins - Post-Op Day Six

Needing prayers.....

Max had another panic attack much as he did yesterday morning. He is completely fed up with the breathing tube and he tries to push it out with his tongue choking himself and then he panics. It was extremely scary to see.

As a result of the attack he is again very sedated, an x-ray will be done shortly to see if the coughing/choking fit actually opened his lungs. Perhaps a positive from a negative?

He also had some bluing of his hands towards the end of the attack so cardiology has ordered an echo cardiogram to be certain that there isn't anything else going on.

Please pray for Max, that his lungs will open and that his heart is fine. We need to get off this roller coaster!

Hopkins - Post-Op Day Five

Today has been a typical Sunday in a hospital....nothing happens!

They have decided not to do a lavage because this morning's x-ray looked much better, in fact there was talk of extubating Max later today. We're not sure if we believe this or not as no one is presently doing anything to meet that goal (again typical Sunday in a hospital). I really like tonight's attending so we're waiting to talk to her.

Speaking of liking caregivers, today has been a challenge. Michele and I changed shifts at about 7 AM when I went home to sleep. Not much later he started to text and call me that Max was having a rough time based mostly on today's nurse who seemed to be less than gentle with him sending his heart rate and blood pressure soaring. Very little of a "plan of action" is being shared with us today, hence my waiting for the night shift.

I'll update later if anything does happen....stay tuned......

Hopkins - Post-Op Day Four

The theme for today was "status quo"....sort of. Max looks great and seems very comfortable although still very sleepy. We have asked them to back down a bit on the pain medication to see if that helps with the grogginess.

Although Max looks so good his chest x-rays tell another story, his left lung is collapsing again. Each of the three or so x-rays taken in the last 24 hours show that although better than where he was yesterday, Max is progressing in the wrong direction. Unless tonight and/or tomorrow morning's films look better they will have to do another bronchoalveolar lavage. Once that's done we will have to wait at least another 24 hours to see if the lung remains inflated. If not there's another less than fabulous theory as to why Max's lung cannot remain open on its own. After speaking with the cardiac surgeon this evening he explained that it is not unusual for patients having repeat cardiac surgeries to have issues with their diaphragms. This is mostly due to the amount of scar tissue produced by the body each time the chest is opened and closed for surgery. It is possible that while they were carefully working through this tissue on Tuesday either a small nerve or the diaphragm itself was nicked. If this is the case it will mean a second surgery to alleviate this problem. As I said, less than fabulous.....

Tonight's prayers are for Max's left lung to open and remain open on its own or at least after a second lavage and therefore a second surgery will not be necessary. Stay tuned....

Hopkins - Post-Op Day Three - 2

Today's bronchoalveolar lavage was a HUGE success! Trying not to offend anyone's sensibilities but they got some super-sized boogers out of Max's left lungs. I have NEVER been so happy to see mucous in my entire life!

By the afternoon Max's chest x-ray showed incredible improvement, much of the collapsed lung was clear. Obviously Max hates the tube but once he woke up from the massive drugs they gave him for the procedure he looked much, much more like his old self! Example below:

Mr. Max makes a return!

Keep praying and stay tuned......

Hopkins - Post-Op Day Three - 1

The roller coaster has gone completely off track. Overnight Max's ability to breathe, even on bi pap, became increasingly difficult if not impossible. He was taken off bi pap and reintubated, he is once again on the ventilator.

The working theory is that after much suctioning a mucous plug must have lodged somewhere causing the left lung to completely collapse. Pulmonolgy has been called and at some point today they will do a bronchoalveolar lavage (literally they will internally "wash" his lungs) at the bedside. The hope is that this procedure will remove copious quantities of mucous allowing the lung to reinflate.

There are a number of other things going on but right now the lungs are obviously the number one priority. We're asking everyone to pray for Max and the healing of his lungs because without this he will not be able to recover overall.

I will try to update later, hopefully with better news. Pray and stay tuned......

Hopkins - Post-Op Day Two

It's been a long day, a very long day. The roller coaster ride continues.

This morning other than being extremely groggy it seemed that Max's respiratory issues were on the mend so at around noon Michele and I left Max with my aunt to go home to sleep and shower. It seemed we barely shut our eyes when my aunt called to say that Max was back in trouble and they were switching him to another type of ventilator. In a little more than an hour we went from almost completely weaned off of oxygen to bi pap at full force.

We did lose a few lines and tubes but we also regained some of them. The classic one step forward two steps back dance. One of the doctors tried to reassure me telling me that I'm an old pro at this and I know that these things can happen. I responded that she was right but I am now ten years older and I just don't have this in me anymore.

So here we sit, Max has spent most of the day asleep or in pain or both. We're praying fiercely for an uneventful night, Max is worn out and scared and he needs a chance to rest and catch up.

Stay tuned.....

Hopkins - Post-Op Day One

The roller coaster ride has officially begun, this ride is far too familiar and not much fun.

In a nutshell Max had a pretty OK night which in the big picture is not bad after open heart surgery. Most of his numbers, i.e., lab work, vital signs, etc. looked good. There were a few things that needed "tweaking" but there always is. Max spent most of the night decidedly NOT knocked out as he should have been. No matter how much pain medication they threw at him he continued to fight the breathing tube. He and I spent a good part of the night playing a game of charades as he tried desperately to talk to me over the tube. As many of you can guess the two words he mostly mouthed to me were "hungry" and "water". Classic Max.

By the time they decided to take him off the ventilator early this morning the drugs decided to kick in and he was just too sleepy to breathe on his own. However, when his surgeon came in mid-morning Max was ready to go and out came the tube.

Here's where the ride begins. Once the tube came out Max was doing well enough for me to leave Michele in charge for a bit while I ran home to take a shower.....very short lived. Max has a pretty regular routine after being extubated and he decided to follow that routine once again. His left lung is now almost completely collapsed and he is needing a decent amount of support to keep his respiratory numbers more or less where they should be. As you can imagine he looks miserable and now needs pain medication. Of course there is a very intricate dance that takes place when mixing narcotics, poor breathing and a need to "cough things up". We need to give Max enough to make him comfortable and willing to cough but not so much as to suppress his breathing.

All of this is part of the "ride". The good news is that everyone is working as a team and we are still very hopeful that we can move past "stable" and get back to "improving". After all, Max and I need to get out of the PICU and to our private room quickly. Neither of us share space well!

So today's prayers are for better breathing, re-inflation of his left lung, and to hold off the low grade temp he seems to be running.

Thanking everyone for following us and continuing to pray....stay tuned.....

Surgical Update - 7 in the PICU (10:45 PM)

Max is now in the PICU, his blood work and vital signs are all pretty much where they want them to be. At this point the things to watch are: keeping his blood pressure low, slowing the bleeding, and keeping his pain in check. Unfortunately Max "knows" their drugs and has a high level of tolerance, in fact at this point they've stopped administering the pain meds "as needed" and changed over to a constant drip. The joke has become that Max will win all the drinking games in college because it takes A LOT to "intoxicate" him.

Tonight we're praying for his vitals, lab work, and drainage tubes to all remain in normal parameters and that he can rest comfortably which will all lend itself to him getting off the ventilator tomorrow. In the cardiac surgery world every tube, every line, every everything they remove from your body puts you one step closer to the door.

I will update tomorrow....stay tuned.....

Surgical Update - 6 (7:00 PM)

We just spoke to the surgeon. There is good news, bad news. The good news is they have closed up and they should be wheeling him by momentarily.

The bad news is that there was a second aortic aneurysm that needed to be grafted, so he now has a graft connected to a graft connected to a mechanical valve. The bionic man in the making. What worries us here is that with Max's "unknown" syndrome we just don't know if these aneurysms will continue to need surgical attention. No one can predict just how much of an aorta can be grafted.

The other concern is that they did have to cool the body to the point where his brain was not being perfused, we had hoped to skip this part because it is always a risk.

All in all things seem to have gone well, the surgeon is happy and therefore so are we!

Stay tuned......

Surgical Update - 5 (6:15 PM)

Max is now off bypass now but is bleeding slightly so they cannot close his chest yet. They are replacing whatever blood is being lost and they are still hopeful that they can be done by 7:30.

Surgical Update - 4 (5:40 PM)

The valve replacement is complete, a small piece of Max's aorta also needed to be repaired as well. His vital signs all remain good and they do not expect there to be much if any bleeding (fingers crossed). The nurse also mentioned that the amount of scar tissue looked good, because Max is a "repeat offender" in the open heart surgery arena they would have expected to see greater scaring.

They are now warming Max's body so that they can take him off of the bypass machine. Now we need to pray for good heart function and no bleeding. Hopefully he'll be completely off the machine in a half hour or so and if all goes well we might be done in about two hours.

Keep praying!

Surgical Update - 3 (3:20 PM)

Heart rate and blood pressure good. Bypass started.

Surgical Update - 2 (2:20 PM)

Surgery has started, Max is doing well.

Surgical Update - 1

About an hour ago we left Max in the OR. As I held the mask over his mouth he began to cry, if I could have I would have picked him up then and there and bolted from the room. Obviously not an option.

After talking with the doctors we have been told to expect an eight to nine hour procedure. We will be updated every two hours or so, as we have news I will post it here.

Max preparing for another "game of his life".

Max and his favorite pre-op nurse.

Hopkins - Day Seven

Specific prayer requests for tomorrow:

1. That Max will not be overly anxious or scared. That he will feel as little pain as possible.




2. That his surgeon, doctors, and nurses will be guided by compassion. That their incredible medical talent and knowledge will be an instrument for another “Max Miracle”.




3. A successful, safe procedure with minimal bleeding and other complications.




4. Minimal time on heart bypass.




5. No long term need for the ventilator or sedation which can often lead to their own complications.




6. And finally please pray for us, eight hours is a long time in a waiting room when you’ve left your son in someone else’s hands…..no matter how wonderful they are at what they do.

I will be updating fairly frequently tomorrow as we get news from the OR so pray there’s only good news to report.

Stay tuned…..

And now a break from our regular programming ......

I just had to share this video clip.

Hopkins - Day Five - The Calm Before the Storm

Finally!! Max at the Air and Space Museum.

If only the whole trip could be like this...... Monkeying around at the Baltimore Zoo.

Hopkins - Day Four

Today was decidedly NOT a good day.

It just started bad and incremented itself into one of those days you wish you could have skipped altogether. There were several things I would categorize as not great news but there were two things in particular that can be categorized as plan old crappy.

First, as a mother I stink....I spent much of this week berating Max for having "made up" his neck pain during our first pre-op appointment. Today we were told that his x-rays from yesterday show a deterioration in his cervical spine, although not uncommon in children with connective tissue disorders it is something we'll need to watch (cause we don't have enough things to watch). It's also something I should not have made lightly of, but who knew? Max never complains that anything hurts so I assumed he was just giving the doctors a hard time.

Second, surgery has been postponed....twice. During our cardiology appointment we were told that a rather sick baby was born last night and needed immediate surgery, obviously this pushed the whole schedule out a day. By the time we saw the surgeon shortly thereafter we had been moved from Tuesday morning to Tuesday afternoon, another sick newborn. This is a good news, bad news situation. The good news is that Max will be the only afternoon case so he will have everyone's FULL attention....definitely good news. Must be an "Italian" thing but this surgeon from Rome seems particularly attentive to Max. More good news. The bad news however, is really bad news. If Max doesn't have surgery until noon we are going to have to keep him away from food for half a day, for everyone who knows Max you can appreciate how painful this will be for all involved.

Stay tuned.....

Hopkins - Day Two

Today was a good day. A very good day.

This afternoon we had our very first appointment with the Hematology clinic here at Hopkins and what was meant to be a run-of-the mill pre-op appointment turned out to be so much more. Max has always had chronic issues with low platelets (the blood cells that cause us to clot when we bleed), obviously a low clotting factor is not a great combination with major surgery. Because of this we assumed that these doctors would probably recommend the obvious, transfuse Max during surgery. But we got so much more! The first doctor in the room was actually a PhD with a background in stem cell research who just so happened to also be a MD (no idiot this guy). He took a lot of time explaining to us that they recently studied and figured out how Max's connective tissue mutation fits in with his low platelet counts. They now understand the entire cell process and will be able to help the surgeons and cardiologists care for Max long term so that they can give him optimum care regarding the necessary blood thinners post valve placement. In other words, the thing that had me the most worried, long term use of blood thinners in an active ten year old boy is no longer quite as scary. I love Hopkins and all their geniuses who are so interested in taking care of and studying Max!! Sometimes it rocks being the only know medical case of something -- doctors find you exciting and trip over each other to be part of your "team".

On Friday morning they will run some very specialized blood work on Max -- in fact this is the only lab in the U.S. (and perhaps the world) that can run these tests and it seems the head of the lab is salivating waiting to "play" with Max's blood!

It never ceases to amaze me how much all these doctors know about Max before we even step foot in their exam rooms. Every time I start to give them his history they stop me to let me know that they've already read everything and have even started their own research. After years and years of hearing, "Oh that's just Max." from so many so called "physicians" who didn't know what else to tell us it seems unreal when these doctors have figured out so much in so little time. They told us they feel as if they are close to having options which will correct Max's platelet issues long term. Have I mention I LOVE HOPKINS? We are so blessed to have found this place were so many are using their God given talents to perform miracles everyday!

It truly was a good day!